Angioedema of the head and neck region T78.3, T78.4, D84.-, T88.7

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Helmut Hentschel

All authors of this article

Last updated on: 29.10.2020

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Definition
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Polyätiologic, acute, single or irregular recurrent, 1-3 day persistent, hereditary or acquired, painful or burning, swelling (edema) of the cutis/subcutis and/or the mucosa/submucosa. Life threatening can be swellings of the upper respiratory tract (pharynx and/or larynx).

Classification
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  • Classification of angioedema of the head and neck region (varies according to Bas et al. 2013)
  • Histamine-mediated angioedema (1%!)
  • non-histamine-mediated angioedema
    • Acute phase mediated angioedema
      • inflammation-induced angioedema - e.g. due to peritonsillar abscess or acute tonsillitis with accompanying inflammatory reactions (about 79%)
      • tumour-induced angioedema (7%)
    • Bradykinin-induced angioedema (8%)
    • idiopathic angioedema (5%)

Clinical features
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Acute phase edema: Inflammatory edema of the head and neck region is the most frequent event in a general consultation, accounting for about 80% of all acute edema in this region. The causes are infectious-inflammatory diseases such as peritonsillar abscesses or acute tonsillitis with concomitant inflammatory oedema. These are painful, mostly unilateral protrusions of the soft palate with an oedema of the uvula.

Bradykinin-induced angioedema: This is the second most frequent group of acute oedema of the head and neck region with 8%. They affect about 30,000 people/year in Germany. Bradykinin-induced angioedema is most often caused by the use of ACE inhibitors. More rarely, angiotensin type I receptor blockers (Sartane) are the cause. Bradykinin-induced angioedema occurs mainly in the first 3 years of intake. However, there are also cases where much longer latency periods (up to 11 years) have been observed.

Histamine-mediated angioedema: mainly in patients with acute or chronic urticaria. Here the clinical appearance leads quickly to the diagnosis. In classic type I reactions, the relationship between exposure and clinical response is usually evident and also memorable to the patient.

Hereditary angioedema (see Angioedema hereditary)

Acquired Angioedema (AAE)

Therapy
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Diagnostic path in acute angioedema of the head and neck region

  • Alerting an Airway team (anaesthetist, emergency doctor)
  • Securing the respiratory tract (oral examination)
  • Where is the angioedema? (tongue, soft palate, hypopharynx/larynx, facial lip)

Therapy of histamine-induced anigoedema

  • Clemastine 2 mg IV.
  • Prednisolone 500 mg IV
  • Adrenaline if necessary with circulatory involvement (0.3-0.5mg adrenaline i.m.)

Therapy of bradykinin-induced angioedema

  • Icatibant 30 mg s.c. (effect after 30 to 45 minutes)
  • alternatively, and still experimentally, C1 inhibitor concentrate (Berinert P, Cinryze, Ruconest) or administration of a kallikrein inhibitor (Kalbitor)
  • if necessary, adrenaline with circulatory involvement (0.3-0.5mg adrenaline i.m.)

Literature
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  1. Bas M et al (2013) Angioedema of the head and neck region. Allergo J 22:118-123
  2. Firazyr. [http://ec.europa.eu/health/documents/community-register/2018/20180426140571/anx_140571_en.pdf EMA SUMMARY OF THE CHARACTERISTICS OF THE MEDICINE]

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Disclaimer

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Last updated on: 29.10.2020